Von Willebrand Disease

Von Willebrand disease (VWD) is the most common inherited cause of abnormal bleeding (haemophilia). There are many different underlying genetic causes, most of which are autosomal dominant. The causes involve a deficiency, absence or malfunctioning of a glycoprotein called von Willebrand factor (VWF). There are three types based on the underlying cause and ranging from type 1 to type 3. Type 3 is the most severe.

 

Presentation

Patients present with a history of unusually easy, prolonged or heavy bleeding:

  • Bleeding gums with brushing
  • Nose bleeds (epistaxis)
  • Heavy menstrual bleeding (menorrhagia)
  • Heavy bleeding during surgical operations

Family history of heavy bleeding or von Willebrand disease is very relevant.

 

Diagnosis

Diagnosis is based on a history of abnormal bleeding, family history, bleeding assessment tools and laboratory investigations. Due to all the underlying causes there is no easy von Willebrand disease test. This can make diagnosis challenging and beyond the scope of most medical exams.

 

Management

Von Willebrand disease does not require day to day treatment. Management is required either in response to major bleeding or trauma (to stop bleeding) or in preparation for operations (to prevent bleeding):

  • Desmopressin can be used to stimulates the release of VWF
  • VWF can be infused
  • Factor VIII is often infused along with plasma derived VWF

Women with VWD that suffer with heavy periods can be managed by a combination of:

  • Tranexamic acid
  • Mefanamic acid
  • Norethisterone
  • Combined oral contraceptive pill
  • Mirena coil

Hysterectomy may be required in severe cases.

 

Last updated April 2019
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